title: Brain microglia were activated in sporadic CJD but almost unchanged in fatal familial insomnia and G114V genetic CJD.
abstract: BACKGROUND
  Microglial activations have been described in different subtypes of human prion diseases such as sporadic Creutzfeldt-Jakob disease (CJD), variant CJD, Kuru and Gerstmann-Sträussler-Scheinker disease (GSS). However, the situation of microglia in other genetic prion diseases such as fatal familial insomnia (FFI) and familial CJD remains less understood. The brain microglia was evaluated comparatively between the FFI, G114V and sCJD cases in the study.
  
  
  METHODS
  Specific Western blots, immunohistochemical and immunofluorescent assays were used to detect the changes of microglia and ELISA tests were used for levels of inflammatory cytokines.
  
  
  RESULTS
  Western blots, immunohistochemical and immunofluorescent assays illustrated almost unchanged microglia in the temporal lobes of FFI and G114V gCJD, but obviously increased in those of sCJD. The Iba1-levels maintained comparable in six different brain regions of FFI and G114V cases, including thalamus, cingulate gyrus, frontal cortex, parietal cortex, occipital cortex and temporal cortex. ELISA tests for inflammatory cytokines revealed significantly up-regulated IL-1β, IL-6 and TNF-α in the brain homogenates from sCJD, but not in those from FFI and G114V gCJD.
  
  
  CONCLUSIONS
  Data here demonstrates silent brain microglia in FFI and G114V gCJD but obviously increased in sCJD, which reflects various pathogenesis of different human prion diseases subtypes.
authors.affiliation: State Key Laboratory for Infectious Disease Prevention and Control, Collaborative Innovation Center for Diagnosis and Treatment of Infectious Diseases (Hangzhou), National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention, Chang-Bai Rd 155, Beijing 102206, People's Republic of China.
authors.forename: Qi
authors.initials: Q
authors.lastname: Shi
authors.forename: Wu-Ling
authors.initials: WL
authors.lastname: Xie
authors.forename: Baoyun
authors.initials: B
authors.lastname: Zhang
authors.forename: Li-Na
authors.initials: LN
authors.lastname: Chen
authors.forename: Yin
authors.initials: Y
authors.lastname: Xu
authors.forename: Ke
authors.initials: K
authors.lastname: Wang
authors.forename: Ke
authors.initials: K
authors.lastname: Ren
authors.forename: Xiao-Mei
authors.initials: XM
authors.lastname: Zhang
authors.forename: Cao
authors.initials: C
authors.lastname: Chen
authors.forename: Jin
authors.initials: J
authors.lastname: Zhang
authors.forename: Xiao-Ping
authors.initials: XP
authors.lastname: Dong
mesh_terms: D015153:Blotting, Western; D001921:Brain / Q000473:pathology; D007562:Creutzfeldt-Jakob Syndrome / Q000276:immunology* / Q000473:pathology*; D016207:Cytokines / Q000378:metabolism*; D016643:Encephalopathy, Bovine Spongiform / Q000276:immunology* / Q000473:pathology*; D004797:Enzyme-Linked Immunosorbent Assay; D006801:Humans; D007150:Immunohistochemistry; D034062:Insomnia, Fatal Familial / Q000276:immunology* / Q000473:pathology*; D017628:Microglia / Q000276:immunology*; D008856:Microscopy, Fluorescence
chemical_list: D016207:Cytokines